Molas G, Ponsot P, Amouyal P, Vallin J, Vitaux J, Paolaggi J A, Potet F
Service d'Anatomie et de Cytologie Pathologiques, Hôpital Beaujon, Clichy.
Gastroenterol Clin Biol. 1990;14(12):1010-4.
Exsudative enteropathy was suspected in a 27-year-old man with lower limb edema, hypoprotidemia and hypoalbuminemia. Gastrointestinal mucosa, kidney, liver, and heart were normal. Laparoscopy showed diffuse small intestine lymphangiectasia. This diagnosis was confirmed by the microscopic examination of several biopsies obtained at laparotomy. Pathological examination of peritoneal, lymph nodes, and liver biopsies showed fibrous thickening of the peritoneum and fibrosis of the lymph nodes. Our patient has been followed for 16 years. Substantial improvement of clinical symptoms was obtained by following a special salt-free diet containing short-chain triglycerides. However biochemical abnormalities have persisted. Exsudative enteropathy due to intestinal lymphangiectasia may be observed in heart and liver diseases as well as in malignant affections of mesenteric lymph nodes. If these conditions are excluded, intestinal lymphangiectasia may be considered as a primitive lymph vessel malformation. The discovery of primitive intestinal lymphangiectasia in an adult cannot be attributed to congenital abnormalities alone. Fibrosis encountered in some cases suggests that an inflammatory process of unknown origin may trigger the onset of intestinal lymphangiectasia.
一名27岁男性出现下肢水肿、低蛋白血症和低白蛋白血症，怀疑患有渗出性肠病。胃肠道黏膜、肾脏、肝脏和心脏均正常。腹腔镜检查显示弥漫性小肠淋巴管扩张。剖腹手术获取的多处活检组织的显微镜检查证实了这一诊断。腹膜、淋巴结和肝脏活检的病理检查显示腹膜纤维性增厚和淋巴结纤维化。该患者已随访16年。通过遵循含短链甘油三酯的特殊无盐饮食，临床症状有显著改善。然而，生化异常仍然存在。肠道淋巴管扩张引起的渗出性肠病可见于心脏和肝脏疾病以及肠系膜淋巴结恶性病变。如果排除这些情况，肠道淋巴管扩张可被视为原发性淋巴管畸形。在成年人中发现原发性肠道淋巴管扩张不能仅归因于先天性异常。某些病例中出现的纤维化提示，不明原因的炎症过程可能引发肠道淋巴管扩张的发生。
